Treatment of Ewing Tumors by Stage

Treatment of a Ewing tumor is based mainly on its location and how much it has spread when first found.

Localized Ewing tumors

As mentioned earlier, even children with localized Ewing tumors often still have cancer spread to other parts of the body that is too small to be seen with imaging tests. If these children do not get chemotherapy, these small pockets of cancer cells would eventually become larger tumors. This is why chemotherapy, which can reach all parts of the body, is an important part of treatment for localized Ewing tumors.

Once the Ewing tumor has been diagnosed and staged, the first treatment is chemotherapy. It is called neoadjuvant chemotherapy because it is given before any surgery or radiation therapy. In the United States, treatment is usually a regimen known as VAdriaC/IE, which is a combination of vincristine, doxorubicin (Adriamycin), and cyclophosphamide, alternated with ifosfamide and etoposide, although other combinations of the same drugs are also effective.

After at least 12 weeks of chemotherapy, imaging tests such as CT, MRI, PET, or bone scans are done to see if the tumor is shrinking and can be surgically removed.

If so, surgery is done at this point. If cancer cells are found at or near the edges of the surgery specimen (meaning cancer cells may have been left behind), radiation therapy and chemotherapy (for several months) are used. If there are no cancer cells at or near the edges of the surgery specimen, chemotherapy can be used without radiation therapy.

If the tumor is not growing but surgery is not an option after the initial chemotherapy, radiation therapy (along with chemotherapy) is usually the next treatment given. In some cases this might shrink the tumor enough so that surgery can then be done. This would then be followed by more chemotherapy, possibly with more radiation as well. In other cases where surgery is still not an option, radiation therapy and chemotherapy are the main treatments.

If the Ewing tumor continues to grow despite the initial chemotherapy, a second type of chemotherapy (using different drugs) may be tried. Surgery or radiation therapy may also be tried to help keep the tumor under control. This may be followed by more chemotherapy.

Metastatic Ewing tumors

Patients who clearly have metastatic disease when they are first diagnosed are harder to treat than patients with localized disease. The outlook is better when the cancer has only spread to the lungs, as opposed to when the cancer has spread to other bones or to the bone marrow.

Treating metastatic disease is similar in many ways to treating localized disease. Chemotherapy is the first treatment, often using a more intense regimen than would be used if the cancer was localized. After a few months, tests such as CT or MRI scans, bone or PET scans, and/or bone marrow biopsies are done to see how the cancer has responded to treatment.

If the cancer has only spread to a few small areas, the main (primary) tumor and all known areas of metastases may be removed with surgery at this point. Other options, such as surgery plus radiation therapy (before and/or after surgery) or radiation therapy alone to all known metastatic sites, including the lungs, might also be tried. During and after these treatments, chemotherapy is given for several months as well.

Doctors at several cancer centers are now studying giving very intensive chemotherapy followed by a stem cell transplant to try to improve the outcome for these patients. For more on this procedure, see “High-dose chemotherapy and stem cell transplant.”

Because these tumors can be hard to treat, clinical trials of newer treatments may be a good option in many cases.

Ewing tumors that recur (come back) after treatment

Recurrence of Ewing tumors after treatment is less likely now than in the past, but it can happen. If the tumor does recur, treatment depends on a number of factors, including:

  • The size and location of the tumor
  • Whether it has spread to different parts of the body
  • What types of treatment were used before
  • How long it has been since treatment

Chemotherapy, surgery, radiation therapy, or some combination of these may be used to treat recurrent tumors, depending on the situation. Doctors are also studying the use of high-dose chemotherapy followed by a stem cell transplant, as well as the use of targeted drugs and immune therapies, but it is not yet clear how useful these are. These tumors can be hard to treat, so clinical trials of newer treatments may be a good option.

The American Cancer Society medical and editorial content team
Our team is made up of doctors and master's-prepared nurses with deep knowledge of cancer care as well as journalists, editors, and translators with extensive experience in medical writing.

Last Medical Review: September 18, 2014 Last Revised: February 4, 2016

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